rare association of schizophrenia and unilateral graves’ disease with contralateral thyroid hemiagenesis in two cases of mccune-albright syndrome
نویسندگان
چکیده
the classical triad of mccune-albright syndrome (mas) consists of polyostotic fibrous dysplasia (fd), skin hyperpigmentation (café-au-lait spots), and endocrine dysfunction, frequently seen in females as precocious puberty. etiology is genetically based and is explained by mosaicism of activating somatic mutations of the alpha-subunit of gs protein. clinical presentation is varied and is dependent on the particular distribution of affected cells, causing a broad spectrum of endocrine and non-endocrine manifestations. typical endocrinopathies are precocious puberty, hyperthyroidism, growth hormone excess, hyperprolactemia, and hypercortisolism. manifestations usually occur during infancy and childhood. we present 2 classical cases of mas with rare association of cerebral and endocrine dysfunction (unilateral graves’ disease with contralateral thyroid hemi agenesis). the first case is an adult onset mas with hyperparathyroidism and schizophrenia; this association is hitherto unreported in literature. literature search showed that mutations in the gsα gene may be associated with the pathogenesis of schizophrenia which is similar to the underlying factor in mas. the second is a child exhibiting classical mas with hyperthyroidism (unilateral graves' disease) which is common but is associated contralateral thyroid hemiagenesis.
منابع مشابه
Rare association of schizophrenia and unilateral Graves’ disease with contralateral thyroid hemiagenesis in two cases of McCune-Albright syndrome
The classical triad of McCune-Albright syndrome (MAS) consists of polyostotic fibrous dysplasia (FD), skin hyperpigmentation (café-au-lait spots), and endocrine dysfunction, frequently seen in females as precocious puberty. Etiology is genetically based and is explained by mosaicism of activating somatic mutations of the alpha-subunit of Gs protein. Clinical presentation is varied and is depend...
متن کاملRare association of schizophrenia and unilateral Graves’ disease with contralateral thyroid hemiagenesis in two cases of McCune-Albright syndrome
The classical triad of McCune-Albright syndrome (MAS) consists of polyostotic fibrous dysplasia (FD), skin hyperpigmentation (café-au-lait spots), and endocrine dysfunction, frequently seen in females as precocious puberty. Etiology is genetically based and is explained by mosaicism of activating somatic mutations of the alpha-subunit of Gs protein. Clinical presentation is varied and is depend...
متن کاملThyroid hemiagenesis with Graves' disease, Graves' ophthalmopathy and multinodular goiter.
Thyroid hemiagenesis is a rare congenital anomaly in which one of the thyroid lobes with or without isthmus fails to develop. Here we present a woman patient with thyroid hemiagenesis, Graves' disease and ophthalmopathy with nodular goiter. Fine needle aspiration biopsy of the dominant nodule was suspicious of malignancy. The patient was referred for surgery for total thyroidectomy. Histopathol...
متن کاملMcCune-Albright syndrome: Report of a case
A 29- year old female with bone pain and history of precocious puberty was referred for bone scintigraphy. On physical examination café au lait macular spots were noted on her neck, buttocks and left leg. Bone scan showed multiple areas of intense increased activity which was in favour of polyostotic fibrous dysplasia. Considering the presence of polyostotic fibrous...
متن کاملMcCune - Albright syndrome Claudia
McCune-Albright syndrome (MAS) is classically defined by the clinical triad of fibrous dysplasia of bone (FD), café-au-lait skin spots, and precocious puberty (PP). It is a rare disease with estimated prevalence between 1/100,000 and 1/1,000,000. FD can involve a single or multiple skeletal sites and presents with a limp and/or pain, and, occasionally, a pathologic fracture. Scoliosis is common...
متن کاملMcCune-Albright syndrome
McCune-Albright syndrome (MAS) is classically defined by the clinical triad of fibrous dysplasia of bone (FD), café-au-lait skin spots, and precocious puberty (PP). It is a rare disease with estimated prevalence between 1/100,000 and 1/1,000,000. FD can involve a single or multiple skeletal sites and presents with a limp and/or pain, and, occasionally, a pathologic fracture. Scoliosis is common...
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عنوان ژورنال:
iranian journal of nuclear medicineناشر: tehran university of medical sciences
ISSN 1681-2824
دوره 23
شماره 2 2015
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